Dupuis C, Rey C, Godart F, Vliers A, Gronnier P
Service de cardiologie infantile, Hôpital cardiologique, Lille.
Arch Mal Coeur Vaiss. 1994 May;87(5):607-13.
The authors report 4 cases of the scimitar syndrome with pulmonary hypertension by stenosis of an abnormally draining right pulmonary vein and they also review the literature. All cases were symptomatic from infancy. The diagnosis was confirmed by catheterisation which showed a significant pressure gradient between the right pulmonary vein and the inferior vena cava, and by angiography which demonstrated the stenosis. None of the treatments proposed (interventional catheterisation with dilatation and eventual implantation of a stent, surgery with treatment of the stenosis and reimplantation of the right pulmonary vein in the left atrium, or pneumonectomy) were satisfactory. However, it is possible that earlier treatment could be effective as changes in the pulmonary vascular bed seem to occur very early in these patients.
作者报告了4例因异常引流的右肺静脉狭窄导致肺动脉高压的弯刀综合征病例，并对相关文献进行了综述。所有病例自婴儿期起就出现症状。通过心导管检查证实了诊断，该检查显示右肺静脉与下腔静脉之间存在显著的压力梯度，血管造影则显示了狭窄情况。所提出的治疗方法（介入性导管扩张术及最终植入支架、手术治疗狭窄并将右肺静脉重新植入左心房或肺切除术）均不尽人意。然而，早期治疗可能有效，因为这些患者的肺血管床似乎在很早的时候就发生了变化。
